医学
淀粉样变性
介绍(产科)
假性肠梗阻
皮肤病科
病理
外科
作者
Leen Nesnas,Iea Alabbasi,David Bunting
标识
DOI:10.1093/bjs/znae318.024
摘要
Abstract Aim This study aims to evaluate treatment strategies for acute presentations of systemic amyloidosis in the gastrointestinal tract. Systemic amyloidosis, identified by the extracellular deposition of β-sheet protein fibrils, often affects the GI tract, particularly in primary (AL) amyloidosis. GI amyloidosis can severely disrupt organ function, requiring prompt and effective treatment. This study focuses on optimizing the management of these acute GI manifestations. Methods 66 year old female presented to ED, with 6 days abdominal pain, distention, vomiting, and not opening her bowels for one week. She had a background of amyloidosis secondary to multiple myeloma on medical treatment. CT scan showed the stomach and the majority of the small bowel are grossly distended with fluid. There appeared to be at least one transition point with faecalis loop of small bowel proximal to it. She then had an emergency laparotomy. Results Intraoperative findings showed abnormal, hardened fecal matter throughout the small bowel. An enterotomy was performed with manual evacuation of the bowel contents. Postoperatively, she required ICU support, intravenous fluids, and empirical antibiotics She had a post-operative ileus with high NG output. This was managed with prokinetic agents including erythromycin, metoclopramide, and prucalopride. She was discharged with home based TPN. Conclusion Management of GI amyloidosis varies by type and presentation, focusing on symptomatic relief and systemic treatment. Prokinetic agents are crucial for dysmotility, and severe cases may require parenteral nutrition. Surgical intervention is necessary for obstructions, uncontrolled haemorrhage, or ischemia. Early identification and appropriate management are essential for improved outcomes.
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